Asthma Masqueraders: Differential Diagnosis in Children and Teens

1. Asthma Masqueraders:Differential Diagnosis in Children and Teens Ngoc Ly, MD, MPH Assistant Professor of Pediatrics Pediatric Pulmonary Medicine UCSF Benioff Children’s Hospital

2. Diagnosing Asthma • No gold standard for diagnosis • Clinical diagnosis • Recurrent respiratory symptoms • Wheeze • Cough • Chest tightness • Breathlessness

4. www.european-lung-foundation.org

5. Symptoms History Exam Spirometric Results Treatment Response

6. No Treatment Response • Poor compliance or adherence • Severe or difficult asthma • Not asthma

7. Case #1 • 16 yo girl with 1 year history of recurrent severe dyspnea and wheezing • Multiple ER visits due to severe respiratory distress • Several hospitalizations • Treated with various asthma therapy • No consistent response to therapy

8. normal

9. Vocal cord dysfunction (VCD) • Trigger by exercise or emotional stress • Young, female • Dyspnea with exercise • Sensation of tightness in throat rather than chest. Loud wheeze/stridor over larynx rather than chest. • Respiratory distress • Typically no bronchodilator response

10. Pediatrics 2007;120(4):860

11. Vocal cord dysfunction • Treatment: speech therapy • Ipratropium may block exercise-induced vocal cord dysfunction • Treat underlying conditions, including anxiety, depression, gastroesophageal reflux disease, and rhinosinusitis

12. Exercise-Induced Dyspnea • Outcomes from142 subjects • Median age 14 years (range, 6-21) • Average duration of symptoms of 30.2 months • Male:Female 0.7:1.0 • 98 patients with prior asthma diagnosis • 101 patients were treated including bronchodilators in 96, inhaled corticosteroids in 35, and systemic corticosteroids in 31. • 82 had at least 1 urgent medical care visit • Symptoms were reproduced during exercise testing in 117 patients Mutasim Abu-Hasan et al., ANNALS OF ALLERGY, ASTHMA & IMMUNOLOGY 2005

13. Etiology for EID Mutasim Abu-Hasan et al., ANNALS OF ALLERGY, ASTHMA & IMMUNOLOGY 2005

14. Exercise Induced Dyspnea • EID not always asthma • Treadmill exercise indicated when • other asthma symptoms not present • Bronchodilator doesn’t prevent EID • Normal baseline lung function • Testing: • Pre- and post-exercise spirometry • Full exercise testing including gas exchange and EKG • Laryngoscopy when indicated

15. Case #2 • 10 yo girl who developed a cough along with her cousin after they went swimming at a public pool • The following day, she was coughing so much at school that she failed her math test • Kept out of school x2 weeks because the cough was disruptive • Presented to the ED twice • Treated with continuous albuterol, followed by inhaled corticosteroids and allergy medications- no improvement after 4 weeks • Valium, codeine, then antibiotics were prescribed-none were effective • Otherwise healthy, pertussis test negative • Does not cough in her sleep

16. Habit Cough • Involuntary, repetitive cough • Loud, dry, “barking” or “honking” • Triggered by infection, irritants, or psychological stressors • Can last for months to years • Disruptive to others-affect school attendance • Cough can be a source of secondary gain but not always • Cough ceases while asleep and usually improves with distraction

17. Habit Cough • Normal physical exam; normal lung function • Opiods, benzodiazepine, and asthma therapy are ineffective • Treatment: suggestive therapy, self-hypnosis, speech therapy

18. Case # 3 • 9 yo boy present with acute cough 1 week and fever x 2 days • Treated with zithromax and albuterol for audible wheeze • 2 weeks later, persistent wheeze, started on oral corticosteroids and inhaled corticosteroids • 1 week later no response • CXR taken

19. Foreign Body Aspiration

20. Case # 4 • 12 yo male • Presenting with 5 days of worsening SOB when lying flat • This progress and was waking him up from sleep • Also wheeze at night • Treated with albuterol and Flovent-no improvement • No SOB during the day, physically active at school • No other complaints • Diagnosed with asthma 2 years prior , using albuterol MDI intermittently • No prior hospitalization

21. Mediastinal Mass

22. Case # 5 • 7 yo boy with cough x 2 years • Harsh, dry, barky cough • Paroxysmal coughing with post-tussive emesis • Awaken from sleep • No response to bronchodilator, corticosteroids, theophylline • Cushingoid from repeated prednisone therapy • Multiple hospitalizations

23. Tracheomalacia Wang CC et al. Ann Thorac Surg. 2010

24. Tracheomalacia • Aortopexy-stitching between aorta and sternum to reduce aortal tension on anterior wall of the trachea • Behavioral-successful in mild cases • Albuterol may worsen symptoms due to smooth muscle relaxation

25. Tracheomalacia • Most common tracheal defect • Expiratory stridor/wheeze • Worse during feeding, crying, or agitation • Improve during sleep • Persistent cough, dyspnea, or tachypnea • Usually resolves by 12 months • R/O vascular compression (ring, sling, anomalous innominate artery)

26. Tracheomalacia

27. Cardiac Wheeze • Cardiac conditions that result in pulmonary artery dilation and/or left atrial enlargement, including large left to right shunts, can compress large airways and cause wheezing. • Left ventricular failure or pulmonary venous outflow obstruction can result in distension of the pulmonary vascular bed, bronchiolar wall edema, increased airway resistance, and wheezing.

28. Cardiac Wheeze • Stridor with large airway compression • Wheezing, tachypnea • Recurrent respiratory infections with significant respiratory distress • Apnea

29. Aspiration Syndromes • Chronic cough and wheeze • Swallowing disorders related to neurologic or muscular dysfunction of the pharynx and/or larynx • Chronic microaspiration can cause significant mucosal edema and inflammation • Tracheoesophageal fistula

30. 6 month old boy, term infant, presenting with chronic cough and wheeze only slightly better with asthma medications. Reflux, gagging and choking with feeds. Case # 7

31. Posterior laryngeal cleft complete cricoid cleft partial cricoid cleft laryngotracheal-esophageal clefts supraglottic

33. Posterior laryngeal cleft • Failure of tracheoesophageal septum development • Chronic cough and wheeze • Respiratory distress with feeds, cyanosis • May present at birth • Aspiration, pneumonia • 6% with TEF have PLC

34. Posterior laryngeal cleft • GERD must be controlled • Type I clefts can sometimes be managed with speech and feeding therapy aimed towards decreasing aspiration • Endoscopic or open repair • Overall mortality 43% • Type IV clefts: 93% mortality

35. Other Causes • Immune deficiency • Cystic fibrosis • Bronchiolitis Obliterans • Interstitial lung diseases

36. Conclusions • Assess • Treat • Reassess if poor response to treatment

37. Go Giants !

38. DIFFERENTIAL DIAGNOSTIC POSSIBILITIES FOR ASTHMA • Upper airway diseases 􀀎 Allergic rhinitis and sinusitis • Obstructions involving large airways 􀀎 Foreign body in trachea or bronchus 􀀎 Vocal cord dysfunction 􀀎 Vascular rings or laryngeal webs 􀀎 Laryngotracheomalacia, tracheal stenosis, or bronchomalacia 􀀎 Enlarged lymph nodes or tumor • Obstructions involving small airways 􀀎 Viral bronchiolitis or bronchiolitis obliterans 􀀎 Cystic fibrosis 􀀎 Bronchopulmonary dysplasia 􀀎 Cardiac disease • Other causes 􀀎 Recurrent cough not due to asthma-habit cough 􀀎 Aspiration from swallowing mechanism dysfunction or gastroesophageal reflux

39. ASTHMA MANAGEMENT Clinical Assessment Pharmacologic Therapy Comorbid Conditions Education ICS +/- Other agents Spirometry & Allergy testing Triggers Techniques Action plan Response? Continue Rx Close F/U Assess compliance/technique + - Minimum effective dose Step-up Therapy Consider further evaluation Referral -

40. History • Birth history • Age of onset • Progression • Recurrent infections • Relation to sleep • Severe dyspnea, cyanosis, apnea • Eating or feeding difficulties, reflux, FTT • GI symptoms • Cardiac abnormalities

41. Cough that goes away with sleep Persistent productive cough Prominent dizziness, light-headedness, peripheral tingling Dyspnea with exercise Persistent tachypnea Symptoms

42. Normal exam when symptomatic Tachypnea without wheeze Severe dyspnea without wheeze Stridor or loud inspiratory wheeze Spirometry normal or inconsistent with airflow obstruction when symptomatic Exam

43. Case # 5 3 month old girl with noisy breathing, worse when she is agitated or excited, and especially with colds. Normal birth history.Growing and thriving

44. Tracheomalacia • Most common tracheal defect • Expiratory stridor/wheeze • Worse during feeding, crying, or agitation • Improve during sleep • Persistent cough, dyspnea, or tachypnea • Usually resolves by 12 months • R/O vascular compression (ring, sling, anomalous innominate artery)

45. Double aortic arch Arches posterior to esophagus and anterior to trachea

46. Pulmonary Sling Aberrant left pulmonary artery which arises from right pulmonary artery and crosses between esophagus and trachea

47. Bronchomalacia • Wheezing can present at birth • More commonly becomes apparent in the first two to three months after birth • The wheezing usually becomes more pronounced with activity or in the setting of upper respiratory tract infections • Albuterol may worsen symptoms due to smooth muscle relaxation

48. Case # 7 • 5 month old female infant with Pierre-Robin Sequence, stridor, mild laryngomalacia, FTT, significant reflux • Severe OSA on a sleep study • Chronic cough x 2 months • No gagging or choking with feeds • Bilateral wheeze and crackles on exam